We report a case of aggressive ‘nasal type’ natural killer (NK)/T cell lymphoma initially presenting as a testicular tumor in a Korean man, which quickly took a fatal course by widespread dissemination. Histologically, the testicular mass showed a diffuse dense infiltrate of medium-sized and atypical large lymphoid cells with angiocentric and angiodestructive infiltration and areas of coagulative necrosis on hematoxylin-eosin stained sections. Immunophenotyping by immunohistochemistry yielded surface markers consistent with NK/T cell lymphoma. The Epstein-Barr virus genome was detected by in situ hybridization. During involved-field irradiation and chemotherapy following radical orchiectomy, the tumor disseminated shortly to the skin and soft tissue of his anterior chest wall and central nervous system (CNS). Identical lymphoid infiltrates were present in the patient’s skin. CNS involvement was interpreted as having a leptomeningeal seeding. To the best of our knowledge, this is the 9th reported case of confirmed NK/T cell lymphoma arising from the testis. Relevant literature is reviewed, and the clinicopathologic features, natural history, and treatment options for primary testicular NK/T cell lymphoma are discussed.

Keywords:
Testicular lymphoma, NK/T cell lymphoma
1.
Jaffe ES, Chan JKC, Su IJ, Frizzera G, Mori S, Feller AC, Ho FCS: Report of the workshop on nasal and related extranodal angiocentric T/natural killer cell lymphomas: Definitions, differential diagnosis and epidemiology. Am J Surg Pathol 1996;20:103–111.
2.
Harris NL, Jaffe ES, Stein H, Banks PM, Chan JK, Cleary ML, Delsol G, DeWolf-Peeters C, Falini B, Gatter KC: A revised European-American classification of lymphoid neoplasms: A proposal from the International Lymphoma Study Group. Blood 1994;84:1361–1392.
3.
Society for hematology program. Am J Surg Pathol 1997;21:114–121.
4.
Sun T, Brody J, Susin M, Marino J, Teichberg S, Koduru P, Hall WH, Urmacher C, Hajdu SI: Aggressive natural killer cell lymphoma/leukemia. A recently recognized clinicopathologic entity. Am J Surg Pathol 1993;17:1289–1299.
5.
Chan JKC, Tsang WYW, Lau WH, Cheung MMC, Ng WF, Yuen WC, Ng CS: Aggressive T/natural killer cell lymphoma presenting as testicular tumor. Cancer 1996;77:1198–1205.
6.
Güler G, Altinock G, Üner AH, Sungur A: CD56+ lymphoma presenting as a testicular tumor. Leuk Lymphoma 1999;36:207–211.
7.
Bartolomé J, González-Arenas MC, Menarguez J, Gil J: Linforma de celulas T/NK de tipo nasal de presentacion testicular (letter). Med Clin 2000;114:318.
8.
Pérez-Vallés A, Sabater-Marco V, Carpio-Máñez D, Botella-Estrada R, Nogueira-Vázquez E, Martorell-Cebollada M: Extranodal peripheral T-cell lymphoma with angiocentric growth pattern and Epstein-Barr viral DNA associated affecting paratesticular soft tissue. J Cutan Pathol 2000;27:80–85.
9.
de Bruin PC, Jiwa M, Oudejans JJ, van der Valk P, van Heerde P, Sabourin JC, Csanaky G, Gaulard P, Noorduyn AL, Willemze R, Meijer CJLM: Presence of Epstein-Barr virus in extranodal T-cell lymphomas: Differences in relation to site. Blood 1994;6:1612–1618.
10.
Petrella T, Delfau-Larue MH, Caillot D, Morcillo JL, Casanovas O, Portier H, Gaulard P, Farcet JP, Arnould L: Nasopharyngeal lymphoma: Further evidence for a natural killer cell origin. Hum Pathol 1997;27:827–833.
11.
Chan JKC, Sin VC, Wong KF, Ng CS, Tsang WYW, Chan CH, Cheung MMC, Lau WH: Nonnasal lymphoma expressing the natural killer cell marker CD56: A clinicopathologic study of 49 cases of an uncommon aggressive neoplasm. Blood 1997;89:4501–4513.
12.
de Bruin PC, Kummer JA, van der Valk P, van Heerde P, Kluin PM, Willemze R, Ossenkoppele GJ, Radaszkiewicz T, Meijer CJ: Granzyme B-expressing peripheral T-cell lymphomas: Neoplastic equivalents of activated cytotoxic T cells with preference for mucosa-associated lymphoid tissue localization. Blood 1994;84:3785–3791.
13.
Martenson JA, Buskirk SJ, Ilstrup DM, Banks PM, Evans RG, Colgan JP, Earle JD: Patterns of failure in primary testicular non-Hodgkin’s lymphoma. J Clin Oncol 1988;6:297–302.
14.
Drenou B, Lamy T, Amiot L, Fardel O, Caulet-Maugendre S, Sasportes M, Diebold J, Le Prise PY, Fauchet R: CD3-CD56+ non-Hodgkin’s lymphomas with an aggressive behavior related to multidrug resistance. Blood 1997;89:2966–2974.
15.
Liang R, Chen F, Lee CK, Kwong YL, Chim CS, Yau CC, Chiu E: Autologous bone marrow transplantation for primary nasal T/NK cell lymphoma. Bone Marrow Transplant 1997;19:91–93.
16.
Takenaka K, Shinagawa K, Yoshinobu M, Maeda Y, Makita M, Kozuka T, Ashiba A, Yamamoto K, Fujii N, Nawa Y, Hiramatsu Y, Sunami K, Ishimaru F, Yoshimo T, Kiura K, Harada M: High-dose chemotherapy with hematopoietic stem cell transplantation is effective for nasal and nasal-type CD56+ natural killer cell lymphomas. Leuk Lymphoma 2001;42:1297–1303.
© 2003 S. Karger AG, Basel
2003
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.